口腔及下肢溃疡作为肉芽肿性多血管炎的首发表现
Oral and Lower Extremity Ulcers as the Initial Presentation of Granulomatosis with Polyangiitis.
作者信息
Al Salihi Mohammed Omar, Dominguez Bianca, Mohanlal Viresh, Carlan S J
机构信息
Department of Internal Medicine, Orlando Regional Healthcare, Orlando, Florida, USA.
Division of Nephrology, Orlando Regional Healthcare, Orlando, Florida, USA.
出版信息
Case Rep Med. 2022 Aug 31;2022:2737242. doi: 10.1155/2022/2737242. eCollection 2022.
BACKGROUND
Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis characterized by lung and kidney involvement. It is typically a disease of white females and has a poor prognosis with the average life expectancy of 5 months for a patient without treatment. Oral and skin ulcers are considered to be rare presentations.
CASE
A 39-year-old black male presented to the hospital with oral and skin ulcers and was diagnosed with GPA based on the biopsies of both cutaneous lesions and kidney. He was started on rituximab with minimal improvement. Later he was admitted to the ICU and had plasmapheresis, and he gradually improved and was discharged home 8 days after admission.
CONCLUSION
GPA is an aggressive vascular disorder resulting in possible organ system damage and failure. The role of the sickle cell trait in this patient is undefined, but this combination of gender, race, and presenting symptoms in GPA is extremely unusual.
背景
肉芽肿性多血管炎（GPA）是一种以肺部和肾脏受累为特征的小血管血管炎。它通常是白人女性的疾病，预后较差，未经治疗的患者平均预期寿命为5个月。口腔和皮肤溃疡被认为是罕见的表现形式。
病例
一名39岁的黑人男性因口腔和皮肤溃疡入院，根据皮肤病变和肾脏活检被诊断为GPA。他开始使用利妥昔单抗治疗，但改善甚微。后来他被收入重症监护病房并进行了血浆置换，病情逐渐好转，入院8天后出院。
结论
GPA是一种侵袭性血管疾病，可能导致器官系统损害和功能衰竭。镰状细胞性状在该患者中的作用尚不清楚，但这种性别、种族和GPA表现症状的组合极为罕见。
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